The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry

PRINTO and the Eurofever registry

Research output: Contribution to journalArticle

Abstract

Objectives: Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry.

Methods: A web-based registry collected retrospective data on patients affected by CRMO/CNO. Both paediatric and adult centres were involved.

Results: Complete baseline information on 486 patients was available (176 male, 310 female). The mean age of onset was 9.9 years. Adult onset (>18 years of age) was observed in 31 (6.3%) patients. The mean time from disease onset to final diagnosis was 1 year (range 0-15). MRI was performed at baseline in 426 patients (88%), revealing a mean number of 4.1 lesions. More frequent manifestations not directly related to bone involvement were myalgia (12%), mucocutaneous manifestations (5% acne, 5% palmoplantar pustulosis, 4% psoriasis, 3% papulopustular lesions, 2% urticarial rash) and gastrointestinal symptoms (8%). A total of 361 patients have been treated with NSAIDs, 112 with glucocorticoids, 61 with bisphosphonates, 58 with MTX, 47 with SSZ, 26 with anti-TNF and 4 with anakinra, with a variable response.

Conclusion: This is the largest reported case series of CNO patients, showing that the range of associated clinical manifestations is rather heterogeneous. The study confirms that the disease usually presents with an early teenage onset, but it may also occur in adults, even in the absence of mucocutaneous manifestations.

Original languageEnglish
Number of pages9
JournalRheumatology (Oxford, England)
DOIs
Publication statusPublished - Mar 27 2018

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Registries
Osteomyelitis
Interleukin 1 Receptor Antagonist Protein
Bone and Bones
Myalgia
Acne Vulgaris
Diphosphonates
Non-Steroidal Anti-Inflammatory Agents
Chronic recurrent multifocal osteomyelitis
Exanthema
Age of Onset
Psoriasis
Glucocorticoids
Demography
Pediatrics

Cite this

@article{4a05ae67840945e7961df7d60259d176,
title = "The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry",
abstract = "Objectives: Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry.Methods: A web-based registry collected retrospective data on patients affected by CRMO/CNO. Both paediatric and adult centres were involved.Results: Complete baseline information on 486 patients was available (176 male, 310 female). The mean age of onset was 9.9 years. Adult onset (>18 years of age) was observed in 31 (6.3{\%}) patients. The mean time from disease onset to final diagnosis was 1 year (range 0-15). MRI was performed at baseline in 426 patients (88{\%}), revealing a mean number of 4.1 lesions. More frequent manifestations not directly related to bone involvement were myalgia (12{\%}), mucocutaneous manifestations (5{\%} acne, 5{\%} palmoplantar pustulosis, 4{\%} psoriasis, 3{\%} papulopustular lesions, 2{\%} urticarial rash) and gastrointestinal symptoms (8{\%}). A total of 361 patients have been treated with NSAIDs, 112 with glucocorticoids, 61 with bisphosphonates, 58 with MTX, 47 with SSZ, 26 with anti-TNF and 4 with anakinra, with a variable response.Conclusion: This is the largest reported case series of CNO patients, showing that the range of associated clinical manifestations is rather heterogeneous. The study confirms that the disease usually presents with an early teenage onset, but it may also occur in adults, even in the absence of mucocutaneous manifestations.",
author = "{PRINTO and the Eurofever registry} and Hermann Girschick and Martina Finetti and Francesca Orlando and Susanne Schalm and Antonella Insalaco and Gerd Ganser and Susan Nielsen and Troels Herlin and Isabelle Kon{\'e}-Paut and Silvana Martino and Marco Cattalini and Jordi Anton and Al-Mayouf, {Sulaiman M} and Michael Hofer and Pierre Quartier and Christina Boros and Jasmin Kuemmerle-Deschner and {Pires Marafon}, Denise and Maria Alessio and Tobias Schwarz and Nicolino Ruperto and Alberto Martini and Annette Jansson and Marco Gattorno",
year = "2018",
month = "3",
day = "27",
doi = "10.1093/rheumatology/key058",
language = "English",
journal = "Rheumatology",
issn = "1462-0324",
publisher = ". Published by Oxford University Press on behalf of the British Society for Rheumatology",

}

TY - JOUR

T1 - The multifaceted presentation of chronic recurrent multifocal osteomyelitis

T2 - a series of 486 cases from the Eurofever international registry

AU - PRINTO and the Eurofever registry

AU - Girschick, Hermann

AU - Finetti, Martina

AU - Orlando, Francesca

AU - Schalm, Susanne

AU - Insalaco, Antonella

AU - Ganser, Gerd

AU - Nielsen, Susan

AU - Herlin, Troels

AU - Koné-Paut, Isabelle

AU - Martino, Silvana

AU - Cattalini, Marco

AU - Anton, Jordi

AU - Al-Mayouf, Sulaiman M

AU - Hofer, Michael

AU - Quartier, Pierre

AU - Boros, Christina

AU - Kuemmerle-Deschner, Jasmin

AU - Pires Marafon, Denise

AU - Alessio, Maria

AU - Schwarz, Tobias

AU - Ruperto, Nicolino

AU - Martini, Alberto

AU - Jansson, Annette

AU - Gattorno, Marco

PY - 2018/3/27

Y1 - 2018/3/27

N2 - Objectives: Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry.Methods: A web-based registry collected retrospective data on patients affected by CRMO/CNO. Both paediatric and adult centres were involved.Results: Complete baseline information on 486 patients was available (176 male, 310 female). The mean age of onset was 9.9 years. Adult onset (>18 years of age) was observed in 31 (6.3%) patients. The mean time from disease onset to final diagnosis was 1 year (range 0-15). MRI was performed at baseline in 426 patients (88%), revealing a mean number of 4.1 lesions. More frequent manifestations not directly related to bone involvement were myalgia (12%), mucocutaneous manifestations (5% acne, 5% palmoplantar pustulosis, 4% psoriasis, 3% papulopustular lesions, 2% urticarial rash) and gastrointestinal symptoms (8%). A total of 361 patients have been treated with NSAIDs, 112 with glucocorticoids, 61 with bisphosphonates, 58 with MTX, 47 with SSZ, 26 with anti-TNF and 4 with anakinra, with a variable response.Conclusion: This is the largest reported case series of CNO patients, showing that the range of associated clinical manifestations is rather heterogeneous. The study confirms that the disease usually presents with an early teenage onset, but it may also occur in adults, even in the absence of mucocutaneous manifestations.

AB - Objectives: Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry.Methods: A web-based registry collected retrospective data on patients affected by CRMO/CNO. Both paediatric and adult centres were involved.Results: Complete baseline information on 486 patients was available (176 male, 310 female). The mean age of onset was 9.9 years. Adult onset (>18 years of age) was observed in 31 (6.3%) patients. The mean time from disease onset to final diagnosis was 1 year (range 0-15). MRI was performed at baseline in 426 patients (88%), revealing a mean number of 4.1 lesions. More frequent manifestations not directly related to bone involvement were myalgia (12%), mucocutaneous manifestations (5% acne, 5% palmoplantar pustulosis, 4% psoriasis, 3% papulopustular lesions, 2% urticarial rash) and gastrointestinal symptoms (8%). A total of 361 patients have been treated with NSAIDs, 112 with glucocorticoids, 61 with bisphosphonates, 58 with MTX, 47 with SSZ, 26 with anti-TNF and 4 with anakinra, with a variable response.Conclusion: This is the largest reported case series of CNO patients, showing that the range of associated clinical manifestations is rather heterogeneous. The study confirms that the disease usually presents with an early teenage onset, but it may also occur in adults, even in the absence of mucocutaneous manifestations.

U2 - 10.1093/rheumatology/key058

DO - 10.1093/rheumatology/key058

M3 - Article

C2 - 29596638

JO - Rheumatology

JF - Rheumatology

SN - 1462-0324

ER -